To date, genetic changes in three families of potassium channels have been observed in association with PAH: the KCNK3 gene that encodes the channel KCNK3 (or TASK-1) that belongs to the two-pore-domain potassium channels (K2P); the ABCC8 gene that encodes the subunit SUR1 of the ATP-sensitive potassium channel (KATP) that corresponds to the inwardly-rectifying potassium channels (Kir); and the voltage-gated potassium channels (Kv) [60,61,62]. Here, ABCC8 is linked to pulmonary arterial hypertension.